Physicians' perspectives on the diagnosis and management of periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome

被引:30
作者
Manthiram, Kalpana [1 ]
Li, Suzanne C. [2 ]
Hausmann, Jonathan S. [3 ,4 ]
Amarilyo, Gil [5 ,6 ]
Barron, Karyl [7 ]
Kim, Hanna [8 ]
Nativ, Simona [9 ]
Lionetti, Geraldina [10 ]
Zeft, Andrew [11 ]
Goldsmith, Donald [12 ]
Kimberlin, David [13 ,14 ]
Edwards, Kathryn [1 ]
Dedeoglu, Fatma [3 ]
Lapidus, Sivia [9 ]
机构
[1] Vanderbilt Univ, Sch Med, Nashville, TN 37212 USA
[2] Hackensack Univ, Med Ctr, Hackensack, NJ USA
[3] Boston Childrens Hosp, Boston, MA USA
[4] Beth Israel Deaconess Med Ctr, Boston, MA 02215 USA
[5] Schneider Childrens Med Ctr Israel, Petah Tiqwa, Israel
[6] Tel Aviv Univ, Sackler Fac Med, Tel Aviv, Israel
[7] NIAID, NIH, 9000 Rockville Pike, Bethesda, MD 20892 USA
[8] NIAMSD, NIH, Bethesda, MD 20892 USA
[9] Overlook Hosp, Morristown Med Ctr, Goryeb Childrens Hosp, Pediat Rheumatol,Atlantic Hlth Syst, Morristown, NJ 07960 USA
[10] Univ Calif San Francisco, San Francisco, CA 94143 USA
[11] Cleveland Clin, Cleveland, OH 44106 USA
[12] Drexel Univ, Coll Med, Philadelphia, PA 19104 USA
[13] Pediat Infect Dis Soc, Arlington, VA USA
[14] Univ Alabama Birmingham, Birmingham, AL USA
关键词
Autoinflammatory syndrome; Periodic fever; Physician practice patterns; PFAPA; OUTCOMES; CHILDREN; PROFILE;
D O I
10.1007/s00296-017-3688-3
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
To assess the practice patterns of pediatric rheumatology and infectious diseases subspecialists in the diagnosis and treatment of periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome. An online survey assessing diagnostic and treatment approaches was sent to 424 members of the Childhood Arthritis and Rheumatology Research Alliance (CARRA) and 980 members of the Pediatric Infectious Disease Society (PIDS). 277 physicians (123 from CARRA and 154 from PIDS representing 21% of the total membership) completed the survey. To diagnose PFAPA, most respondents agreed that patients must have the following features of the diagnostic criteria: stereotypical fever episodes (95%), asymptomatic intervals between episodes (93%), and normal growth and development (81%). However, 71% of the respondents did not require age of onset < 5 years, 33% did not require regular intervals between episodes, and 79% did not require the concomitant signs of aphthous stomatitis, adenitis, or pharyngitis during episodes as long as episodes were regular. Over half (58%) considered episode resolution with steroids to be diagnostic of PFAPA. Corticosteroids, antipyretics, tonsillectomy, and cimetidine were the most commonly prescribed treatments, while steroids and tonsillectomy were most effective. Subspecialists in pediatric rheumatology and infectious diseases showed limited adherence to the complete published criteria for diagnosing PFAPA suggesting heterogeneity in the characteristics of patients diagnosed with the disorder. These findings emphasize the need to develop consensus diagnostic and treatment guidelines in well-characterized patient populations.
引用
收藏
页码:883 / 889
页数:7
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